Daniel's Story Part Two

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Welcome to part two of Daniel's story - here's where we left off...

- At that point, after knowing Daniel hadn’t slept for nearly 3 days, being told he would probably be blind and unable to ever have the life he had known before, possibly aware of what was happening but unable to communicate, I picked him up out of his hospital bed and rocked him for a very long time and then told him very quietly that if he was too tired he it was ok to stop fighting, that Mummy and Daddy would understand if he wanted to let go.

And so, on we go with the rest of the tale......

- In situations like the one happening with Daniel the mind can play some funny games – the one memory that kept replaying in my head over and over was the Wellington neurologist telling us back in 2001 that she felt Daniel would be one of the MAE children who was no longer having seizures at age 7 – yet here we were with him having turned 7 yrs old the week before with his seizures more critical than ever before, with his short life in real danger of ending.

- A couple of months earlier Richard had given me a gold bangle for my birthday and had not noticed at the time that the inscription had mistakenly read ‘To Susan, love always from Richard Nicole and Jacob’. Obviously an error with the omission of Daniel’s name, but one we had not got around to rectifying before he had become so ill. Sitting on the stairs outside the Intensive Care Unit during a break I can recall staring at the bracelet, and feeling that fate was intervening – the reason Daniel would not be having seizures at age 7 was because he was not going to be with us any longer, and that the jewelers omission had been part of some ‘plan’. Those are the types of scrambled thoughts that I know those in similar situations can relate to - logic being clouded by emotion, with an impending sense of doom in a converse way providing some sense of relief – if that is what was ‘to be’, the agony of dreading it in some way was lifted as it was already seemingly upon us. A bittersweet sense of relief for Daniel was also obviously present – he would not have to struggle or be in pain any longer, or start a new life that would be so different to the one he had previously known.

- The next 3 days on the 2000 mg per day epilim dose saw no seizure relief – as always in the past when Daniel was heavily medicated we started to see a pattern of an increased convulsive component to the seizures, particularly obvious as the drug peaked. Blood levels taken showed toxic levels on peaking, with sub therapeutic trough levels - even on that dreadfully high dose - another illustration of what we had been witnessing in the past with him, an unusual metabolic processing of the AEDs – perhaps part of the reason for the intractability of his seizures to traditional epilepsy medications – and something we had been starting to see as a trait common in quite a few other MAE children.

- Once it became obvious to the hospital staff as well that the very high epilim dose was hindering rather than helping Daniel, an agreement was made to start reducing it down. The dose was halved to 1000 mgs per day on the 8th of December and by the following day he was starting to improve slightly – the seizures were still coming in frequent clusters, sometimes occurring every 2-3 minutes but he was able to be taken off the ventilator, as his lungs were functioning properly again following the infection he had contracted whilst in the coma.

- He was still unable to see us but was starting to respond vocally – albeit inappropriately, for example he nodded his head to every question we asked him followed by the word ‘yes’ with an eerie type of smile. His body movements were uncontrolled, at that point it was unclear whether this was due to seizure activity or from some form of brain damage sustained from the prolonged status periods.

- The decision was made to move him out of Intensive Care to the High Dependency Unit on the neurological ward as his condition was downgraded from critical to serious – he was breathing on his own and was taking very small sips of fluids.

- After 3 weeks in PICU the staff there had come to know Daniel well (some had also known him beforehand from his 3 previous PICU admissions), and most gathered round to farewell him as his bed was wheeled out – he responded to this with repeated bursts of uncontrolled laughing – again, we were unsure if this was seizure activity or resultant abnormal brain function but although totally inappropriate, the laughing was the sweetest sound we had heard in a long time. We didn't really care that it was not intentional, as Daniel’s giggle, from whatever cause (!) is a highly infectious one and Richard and I, along with the Drs and nurses were overcome with laughing as well. With Daniel still blind with no real purposeful functioning, with no inkling of what his or our future held, it was a very surreal situation to be standing there laughing – one of ‘those’ moments I guess.

- On the HDU unit Daniel continued with frequent seizures, by now mostly tonic again in nature, with arm lifts, neck twisting and eye deviation interspersed with longer convulsive ones. They were sometimes occurring every 20 seconds, then he would have a break of 30 or so minutes, then they would start again.

- On the 10th of December, desperate to get some form of ‘normal’ communication from him, we asked his sister Nicole to talk to him about the train set one of the parents in America from our MAE support group had kindly sent him for his 7th birthday present. She described it to him (as he still couldn't see) and said that if he didn't want it, his little brother Jacob might like it instead. That intentional ‘dig’ from her provoked the first purposeful response from him in many weeks – he sat bolt up right (no mean feat with next to no muscle tone left after being in bed for so long!) and very clearly shouted ‘no’. That moment gave us the first inkling of hope that just maybe he could be on the road to recovery.

- Although he was then unable to stop saying ‘no’ over and over again (!), I realized that his word formation was actually clearer than it had been in over 3 years, perfectly pronounced, with none of the slurred, slow ‘benzo’ speech we had come to expect from him. That too gave us hope – if his speech was starting to revert back to ‘pre benzo’ days, despite the relentless seizure activity he was still experiencing – would everything else as well?

- Daniel still had no normal sleep patterns at that stage, his eyes would close for a short while when we assumed he was getting at least some rest, but it wasn't until Dec the 15th that we witnessed him finally sleeping uninterrupted for a 3 hour stretch after 2 exhausting weeks.

- As his seizures were still however totally uncontrolled, with no options seemingly available to us, we had over the previous week asked our hospital to once again enlist the help of the Wellington neurologist who had helped us back in 2001. As Daniel was still too unstable to travel, we were thrilled to hear that she had agreed to fly up to Auckland to examine him and give her opinion on the status of his EEG.

- She arrived on Dec the 16th, and after reviewing his previous EEGs and arranging for a new video EEG that she could witness herself, pronounced him still in tonic status and still therefore in danger.

- She also said that his blindness may be a fairly rare reaction (surprise surprise!) to the thiopentone used to induce the coma, one which may therefore be temporary only, that time would tell.

- As an alternative to the more traditional 'status breaking' medications, she suggested a trial of rapidly incremented topomax which she had seen successfully used elsewhere. Rather than the usual slow incrementing up to a theraputic dose over weeks/months to help reduce inhibitive side effects (time which Daniel obviously did not have at his disposal...), it was started that night at 25 mgs, with an increase of 25 mgs per day planned up to a theraputic level of between 5 and 10 mgs per kg - in Daniel's case between 150 and 250 mgs per day, perhaps even up to 12 mgs per kg or 300 mgs per day if it was showing promise but not full control.

- She also agreed that the 1000 mg epilim dose may be hindering more than helping and recommended halving it to 500 mgs per day. We felt comfortable with this dose as it reduced it back down to the 18-20 mg per kg dose we had always worried about exceeding, especially in light of the fact we had seen a cluster of 7 seizures occur that day as the higher epilim dose had peaked.

- We also changed the dosing times of the epilim, to be taken 3 times a day rather than the previous 2 as this seemed to help with the extreme peaks and troughs Daniel had always experienced with this AED.

- Day 5 after starting the topomax saw an increase in the myoclonic component of the seizures, but we didn't know if this was a paradoxical reaction to the topomax (as this med can sometimes do in predominantly myoclonic syndromes like MAE) or whether it was a withdrawal type reaction to the epilim reduction 5 days earlier so we didn't change anything and continued to watch and wait. That night we realised Daniel was starting to see again - I held up a toy car and asked him what colour it was and he correctly answered 'red', I held a book up in front of him and asked what was on it and he correctly answered 'koala' - so not only was he able to see but he was also answering appropriately - still with some repetitive tendencies, but we knew that night that he had definitely retained basic brain function - to what extent we didn't know, but the relief we felt is pretty hard to put into words. Encouraged by the beginning of his 'return', we helped him walk a few steps round the hospital room - he seemed unsure when to place which foot where (!) and he was very shaky whilst upright but it was great to see him out of his bed and off his back :)

- The following day (Dec 21st) there was no sign of the temporary myoclonic increase seen the day before, and we saw the first 24 hr period without a generalised seizure. That morning we had found out the night nurse had forgotten to give the evening dose of epilim the night before so were unsure whether that had helped (!) or whether the topomax was starting to kick in. It was also now almost 6 weeks since his last dose of the aggravating benzo meds, the time frame we had hoped most of the drugs would have leeched out of his bloodstream and fatty tissues by.

- His blood acidity level had also increased in the previous 24 hrs (presumably from the acid inducing topomax) and this was another factor we had witnessed as a component that seemed to help with his keto diet seizure control, and his ketones were back in a steady range - so all in all there were a lot of changes happening at once, too many to isolate one factor to credit his improvement to.

- The topomax was by this stage at 150 mgs per day, so the very bottom of the theraputic limit, and only at that dose from the night before. Because of the vast improvements we had already started to see, with only a very slim chance that the topomax was wholly responsible, we asked if we could hold off increasing any further for a while, knowing from the past that 'less is always more' with Daniel med wise.

- That night Daniel read 3 lines from a basic story book and recited our phone number and spelt out his name, later spending some time sitting up unaided in an armchair watching television. His head moved from left to right whilst watching the screen rather than just his eyes moving, apparently because his brain function was still slowed and each 'task' he was re-learning needed time to process individually.

- No 'awake' seizures were occurring by this stage, only intermittent sleep seizures so 3 days later we were allowed to take him home on Dec 24th, Christmas Eve, for 24 hrs 'leave' from the hospital. During the morning he had spent time with the hospital physiotherapist practicing his walking - a slow, shaky process, but he had by then 'remembered' how to walk, it was just a matter of supporting him until he fully regained his strength.

- Six weeks of virtual inactivity had taken it's toll on him physically - he had lost a lot of muscle mass, he had required splints on his ankles to try and keep them extended towards the end of his PICU stay, and his fists were often still clenched in a type of dystonic posturing ; but to be able to assist him to the car rather than carry him as we had previously expected to be doing the next time we left the hospital was to us nothing short of a miracle.

- That night at home before bed after he had laid out his Christmas stocking, I took him out onto our balcony to show him all the Christmas lights on the very brightly (!) adorned house across the road and the memory of the pure delight and excitement on his face is still now as clear as that night nearly 4 1/2 years ago. He still had the nasal gastric tube in as he was still adjusting to swallowing solid foods, he was still slow and tentative in a lot of his actions, but his speech was clear, and he was alive and well, a Christmas present for us as a family that I know none of us will ever forget.

- He had three fairly brief seizures that night at about 11.30 PM but none after midnight so there was no post -ictal state when he woke up and he was able to fully enjoy Christmas Day with the family. He was even able to have some small keto meals with only a tube 'top up'. We (reluctantly!) took him back to the hospital late Christmas afternoon with a nurse booked to watch over him until we were able to return the next day - we needed to spend some time with Nicole and Jacob and for the first time since the beginning of November we finally felt able to leave Daniel without one of us being there with him.

- On Boxing Day when we returned to the hospital Daniel was happily absorbed in a play station game with one of the Drs who had last seen him in October looking on in surprise - he had heard about the long admission and the 6 week convulsive status, so was not expecting to see a relatively alert 7 yr old moving effortlessly up the play station game levels when he arrived to do his ward rounds that day :) We too were rather amazed to see the ease with which he was playing, his hand eye co-ordination was obviously on the mend along with everything else....

- We asked how many seizures the nurse had witnessed over night and were told there had been none at all - we were obviously pleased but a bit sceptical as Daniel having none of the ever present sleep seizures was more than we could have hoped for. But that night again there were none seen, nor the next night, and so the three he had just before midnight on Christmas Eve 2003 turned out to be the last ones we have seen.

- Daniel was discharged on New Years Eve 2003 - no seizures, no nasal gastric tube, walking, talking and with a renewed lease on life. His medication doses remained the same as on 21.12.03 - 150 mgs per day of topomax, 500 mgs per day of epilim and the ketogenic diet. That first night of 'normality' in our household, basically since June 2000, especially after the upheaval of the late 03 nightmarish hospital admission was another experience we will never forget - the simple joy of regular play, bath and bed time for the kiddies something that those who have been through a life changing experience such as ours would appreciate!

- Daniel had an EEG in the 2nd week of January 2004, so about 10 days after his discharge and about 3 weeks after we saw his last seizure - there were no signs of any epileptiform activity and the background was completely normal for his age - the Drs had expected to see some sort of slowing after the barrage of seizures from the 6 week convulsive status and even though he was clinically seizure free, they were anticipating some 'spikes' to show as they assumed his brain would still be in the 'calming down' stage. But nope, all clear :)

- We kept Daniel's treatment regime the same for about 6 months, not really believing that the epilim in particular was part of the seizure freedom he continued to experience but unwilling to upset the apple cart - as others will know in that position, wanting to abide by the 'if it ain't broke don't try to fix it' rule....

- However after 3 1/2 yrs of delayed learning due to both the seizure activity and the various drug regimes he had been on, as well as many extended absences, Daniel's schooling had fallen very far behind. We made the decision to take away one of the potentially cognitively inhibitive meds and as we had promised ourselves and the hospital to retain the topomax for a 2 yr seizure free period 'just in case', epilim is the one we chose to withdraw.

- The wean went very smoothly in 2004, with no adverse events occurring. We saw an increase in overall alertness as well as improved motor skills and memory once it had all gone. We knew the topomax (especially because of the rapid fashion with which it was introduced) would be playing a part with some of his learning difficulties but the fact that he was learning and starting to catch up academically as well as being able to absorb new skills gave us hope that eventually one day he may get back to where he had been 'before'.

- We decided to start slowly weaning him off the ketogenic diet in February 2005, 14 mths after his last seizure but didn't let his ratio fall to 2:1 until the 18 mth seizure free mark in June 2005 had passed. The diet wean was fully completed by his 9th birthday in November 2005, celebrated with a 'real' chocolate birthday cake.

- Even now, after being off keto for 3 and a half years, Daniel still absolutely 'rejoices' in food. He eats practically everything offered to him, including every vegetable known to man! Pre keto he was a very picky eater and strictly a carb junkie with corn being the only vege he would eat, so 4 years of restricted diet choices has definitely had a positive influence on his eating habits. I can't think of many times since eating freely that he has returned a plate to us that is not almost clean enough to put straight back in the cupboard! He still literally savours every bite, so is always the last to finish at family mealtimes - I am beginning to think he will always be that way!

- Despite predictions saying otherwise, his growth which was slowed for so long following his 2 catabolic episodes has been catching up over the years, and he has now clawed his way back to the 75th percentile for height from just below the 50th to where it fell - it is doubtful he will ever be back up above the 90th percentile but obviously that is not important - he is not a 'shortie' any more, as he was starting to be called a few years ago (!) and a bit of a dip in the height percentile stakes is a miniscule price to pay for him having his health - and his life - back again.

- In November 2005, after nearly 2 years of seizure freedom we started the topomax wean - we started a couple of months earlier than planned as we wanted him to restart the school year in February 2006 medication free, and the 6 week Christmas break seemed the most logical time to carry out the bulk of it where we could keep a close eye on him and control his activities etc during each reduction.

- That too went without a hitch, and so in Feb 2006, 5 years and 8 months after the seizure nightmare first began in June 2000, we finally had a seizure free, treatment free, son back again.

- Academically we do still see some delays, mainly to do with logic which affects his maths in particular, but his school has been fantastic in their efforts to assist in his rehabilitation - one by one he has been discharged from programs there which focussed on Speech, Occupational therapy, Physiotherapy, Auditory Processing and Remedial Reading. The only extra help he requires there now is one on one Remedial Maths three days a week (topped up by us with a tutor at home here once a week) and he started this school year in February 2008 for the first time without a teacher aide.

- He is now considered fully independent in the classroom and has a wide circle of friends, and was able to go away to school camp recently for a week along with all the other year 6 children.

- Because we now have what should probably be termed a 'renewed lease of life', we have spoilt ourselves a bit with 3 family overseas trips since Daniel's recovery, the first was a trip to the Gold Coast in October 2004 to go to all the beaches and theme parks there (Daniel was still on keto and topomax though which he tended to overheat on so we were a bit restricted in activities etc), then to LA (Disneyland) and Las Vegas in April 2006, and we have just returned from a return trip to the theme parks at the Gold Coast - this time with a 'treatment free' and slightly older Daniel so he was able to enjoy all the activities on offer.

- He has shown himself to be a bit of a thrill seeker with countless rides on roller coasters, huge water slides and all the other ones that I was too petrified to even go near! He absolutely loves any type of wildlife, always has, and the animal enclosures we visited there were probably one of his highlights.

- The icing on the cake for us with this latest trip was our insurance company advising us that we no longer had to apply for approval to cover a pre-existing medical condition for Daniel before we travel - because of the length of time he has been seizure and treatment free for, they now consider his epilepsy to be 'resolved'. I think I am finally at the point of allowing myself to agree - never thought I would be saying that.....

- Morals to come out of our long winded story......do not ever give up hope, even when you think things can really get no worse and trust your instincts when it comes to your children, they are usually right........

Once again, many many thanks to Susan for sharing this story of hope with us. With lots of love from your MF's mates for your continuing involvement - you give us so much useful information, and above all HOPE!